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Cystic biliary atresia with congenital absence of portal vein. A 126-day female child presented with jaundice since day 7 of life. She was icteric and had hepatosplenomegaly. Radiological investigations revealed three extrahepatic and multiple intrahepatic biliary cysts, absent gall bladder and portal vein. These findings were confirmed on laparotomy. To the best of our knowledge, this is the first report of cystic biliary atresia associated with congenital absence of portal vein.